Epidemiology and Survival of Systemic Sclerosis-Systemic Lupus Erythematosus Overlap Syndrome
Epidemiology and Survival of Systemic Sclerosis-Systemic Lupus Erythematosus Overlap Syndrome
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DOI：
10.3899/jrheum.170953
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发表时间：
2018-10-01
影响因子：
3.9
通讯作者：
Johnson, Sindhu R.
中科院分区：
文献类型：
--
作者：
Alharbi, Samar;Ahmad, Zareen;Johnson, Sindhu R.
客观的。系统性硬化症（SSc）可能与系统性红斑狼疮（SLE）重叠。对于 SSc-SLE 重叠的流行病学、临床特征和生存情况知之甚少。我们评估了 SSc-SLE 重叠的发生率和 SSc 特征的差异，并比较了与无 SLE 的 SSc 的生存率。方法。进行了一项队列研究，包括符合美国风湿病学会 (ACR)/欧洲抗风湿病联盟 SSc 分类标准和/或 ACR SLE 标准的受试者。主要结局是从诊断到全因死亡的时间。使用 Kaplan-Meier 和 Cox 比例风险模型评估生存率。结果。我们确定了 1252 名受试者（SSc：n = 1166，SSc-SLE：n = 86），SSc-SLE 患病率为 6.8%。 SSc-SLE 患者诊断时年龄较小（37.9 岁 vs 47.9 岁，p < 0.001），更常见的是东亚人（5.5% vs 20%）或南亚人（5.1% vs 12%），患有狼疮抗凝物（6% vs 03%，p < 0.001）、抗心磷脂抗体（6% vs 0.9%，p < 0.001）和肺动脉高压（PAH；52% vs 31%，p < 0.001）。 SSc-SLE 患者较少出现钙质沉着（13% vs 27%，p = 0.007）、毛细血管扩张（49% vs 75%，p < 0.001）和弥漫性亚型（12% vs 35%，p < 0.001）。肾危象（7% vs 7%）、间质性肺疾病（ILD；41% vs 34%）和指溃疡（38% vs 32%）的发生率没有显着差异。 SSc-SLE 患者的中位生存时间更长（26.1 年 vs 22.4 年），但这并不具有统计学意义（对数秩 p = 0.06）。女性性别和弥漫亚型减弱了组间生存差异（HR 1.07，95% CI 0.67 1.67）。结论。 SSc-SLE 患者诊断时年龄较小，更常见 PAIL，较少出现 SSc 皮肤表现。应监测他们的 ILD、肾危象和指溃疡。
Objective. Systemic sclerosis (SSc) may overlap with systemic lupus erythematous (SLE). Little is known about the epidemiology, clinical characteristics, and survival of SSc-SLE overlap. We evaluated the prevalence of SSc-SLE overlap and differences in SSc characteristics, and compared survival with SSc without SLE.Methods. A cohort study was conducted including subjects who fulfilled the American College of Rheumatology (ACR)/European League Against Rheumatism classification criteria for SSc and/or the ACR criteria for SLE. The primary outcome was time from diagnosis to all-cause mortality. Survival was evaluated using Kaplan-Meier and Cox proportional hazard models.Results. We identified 1252 subjects (SSc: n = 1166, SSc-SLE: n = 86) with an SSc-SLE prevalence of 6.8%. Those with SSc-SLE were younger at diagnosis (37.9 yrs vs 47.9 yrs, p < 0.001), more frequently East Asian (5.5% vs 20%) or South Asian (5.1% vs 12%), had lupus anticoagulant (6% vs 03%, p < 0.001), anticardiolipin antibody (6% vs 0.9%, p < 0.001), and pulmonary arterial hypertension (PAH; 52% vs 31%, p < 0.001). Those with SSc-SLE less frequently had calcinosis (13% vs 27%, p = 0.007), telangiectasia (49% vs 75%, p < 0.001), and diffuse subtype (12% vs 35%, p < 0.001). There were no significant differences in the occurrence of renal crisis (7% vs 7%), interstitial lung disease (ILD; 41% vs 34%), and digital ulcers (38% vs 32%). Those with SSc-SLE had better median survival time (26.1 vs 22.4 yrs), but this was not statistically significant (log-rank p = 0.06). Female sex and diffuse subtype attenuated survival differences between groups (HR 1.07, 95% CI 0.67 1.67).Conclusion. Patients with SSc-SLE are younger at diagnosis, more frequently have PAIL and less frequently have cutaneous manifestations of SSc. They should be monitored for ILD, renal crisis, and digital ulcers.